title: Disease animal models of TDP-43 proteinopathy and their pre-clinical applications.
abstract: Frontotemperal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS) are two common neurodegenerative diseases. TDP-43 is considered to be a major disease protein in FTLD/ALS, but it's exact role in the pathogenesis and the effective treatments remains unknown. To address this question and to determine a potential treatment for FTLD/ALS, the disease animal models of TDP-43 proteinopathy have been established. TDP-43 proteinopathy is the histologic feature of FTLD/ALS and is associated with disease progression. Studies on the disease animal models with TDP-43 proteinopathy and their pre-clinical applications are reviewed and summarized. Through these disease animal models, parts of TDP-43 functions in physiological and pathological conditions will be better understood and possible treatments for FTLD/ALS with TDP-43 proteinopathy may be identified for possible clinical applications in the future.
authors.affiliation: Institute of Clinical Medicine, National Cheng Kung University, Tainan 704, Taiwan. kjtsai@mail.ncku.edu.tw.
authors.forename: Yu-Chih
authors.initials: YC
authors.lastname: Liu
authors.forename: Po-Min
authors.initials: PM
authors.lastname: Chiang
authors.forename: Kuen-Jer
authors.initials: KJ
authors.lastname: Tsai
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000378:metabolism / Q000473:pathology; D000818:Animals; D004268:DNA-Binding Proteins / Q000378:metabolism*; D004195:Disease Models, Animal; D057174:Frontotemporal Lobar Degeneration / Q000378:metabolism / Q000473:pathology; D006801:Humans; D057177:TDP-43 Proteinopathies / Q000378:metabolism* / Q000473:pathology*
chemical_list: D004268:DNA-Binding Proteins